Managing Children with Gaucher Disease: Modern Clinical Recommendations
The focus of this article is Gaucher disease — a rare enough hereditary pathology. The authors present the most up-to-date epidemiological data and features of Gaucher disease etiopathogenesis. They offer clinical characteristics for the various types of this disease. The algorithm and crucial steps...
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| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | Russian |
| Published: |
Union of pediatricians of Russia
2016-08-01
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| Series: | Педиатрическая фармакология |
| Subjects: | |
| Online Access: | https://www.pedpharma.ru/jour/article/view/1423 |
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| author | A. A. Baranov L. S. Namazova-Baranova O. S. Gundobina E. A. Lukina A. K. Gevorkyan K. V. Savostyanov A. A. Pushkov E. A. Vishnyova G. B. Movsisyan |
| author_facet | A. A. Baranov L. S. Namazova-Baranova O. S. Gundobina E. A. Lukina A. K. Gevorkyan K. V. Savostyanov A. A. Pushkov E. A. Vishnyova G. B. Movsisyan |
| author_sort | A. A. Baranov |
| collection | DOAJ |
| description | The focus of this article is Gaucher disease — a rare enough hereditary pathology. The authors present the most up-to-date epidemiological data and features of Gaucher disease etiopathogenesis. They offer clinical characteristics for the various types of this disease. The algorithm and crucial steps of differential diagnosis are described in detail. Also, the tactic and algorithms of enzymereplacing therapy (pathogenetic treatement of this hereditary enzymopathy) are carefully presented, together with the modern scheme of managing patients according to the corresponding health care delivery stages. |
| format | Article |
| id | doaj-art-83d96af2192e4d659928b9f95e6ea423 |
| institution | Kabale University |
| issn | 1727-5776 2500-3089 |
| language | Russian |
| publishDate | 2016-08-01 |
| publisher | Union of pediatricians of Russia |
| record_format | Article |
| series | Педиатрическая фармакология |
| spelling | doaj-art-83d96af2192e4d659928b9f95e6ea4232025-08-20T03:39:29ZrusUnion of pediatricians of RussiaПедиатрическая фармакология1727-57762500-30892016-08-0113324425010.15690/pf.v13i3.15741408Managing Children with Gaucher Disease: Modern Clinical RecommendationsA. A. Baranov0L. S. Namazova-Baranova1O. S. Gundobina2E. A. Lukina3A. K. Gevorkyan4K. V. Savostyanov5A. A. Pushkov6E. A. Vishnyova7G. B. Movsisyan8Scientific Center of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian Federation Pirogov Russian National Research Medical University, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian FederationHematology Scientific Center, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian Federation Sechenov First Moscow State Medical University, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian FederationScientific Center of Children’s Health, Moscow, Russian Federation Pirogov Russian National Research Medical University, Moscow, Russian FederationThe focus of this article is Gaucher disease — a rare enough hereditary pathology. The authors present the most up-to-date epidemiological data and features of Gaucher disease etiopathogenesis. They offer clinical characteristics for the various types of this disease. The algorithm and crucial steps of differential diagnosis are described in detail. Also, the tactic and algorithms of enzymereplacing therapy (pathogenetic treatement of this hereditary enzymopathy) are carefully presented, together with the modern scheme of managing patients according to the corresponding health care delivery stages.https://www.pedpharma.ru/jour/article/view/1423gaucher diseasestorage diseaseslipid metabolismetiologypathogenesisdifferential diagnosisclinical coursetreatmentenzyme-replacing therapychildren |
| spellingShingle | A. A. Baranov L. S. Namazova-Baranova O. S. Gundobina E. A. Lukina A. K. Gevorkyan K. V. Savostyanov A. A. Pushkov E. A. Vishnyova G. B. Movsisyan Managing Children with Gaucher Disease: Modern Clinical Recommendations Педиатрическая фармакология gaucher disease storage diseases lipid metabolism etiology pathogenesis differential diagnosis clinical course treatment enzyme-replacing therapy children |
| title | Managing Children with Gaucher Disease: Modern Clinical Recommendations |
| title_full | Managing Children with Gaucher Disease: Modern Clinical Recommendations |
| title_fullStr | Managing Children with Gaucher Disease: Modern Clinical Recommendations |
| title_full_unstemmed | Managing Children with Gaucher Disease: Modern Clinical Recommendations |
| title_short | Managing Children with Gaucher Disease: Modern Clinical Recommendations |
| title_sort | managing children with gaucher disease modern clinical recommendations |
| topic | gaucher disease storage diseases lipid metabolism etiology pathogenesis differential diagnosis clinical course treatment enzyme-replacing therapy children |
| url | https://www.pedpharma.ru/jour/article/view/1423 |
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